Dermatofibrosarcoma protuberans with unusual presentation in vulva

Gynecological sarcomas are rare and their location in the vulva and vagina has an incidence of 5% of all malignant neoplasms in the female genital tract. We present the case of a 54-year-old patient with a diagnosis of dermatofibrosarcoma protuberans in the vulva, an infrequent pathology with less than 60 cases reported worldwide in this anatomical location. Clinically, it is locally aggressive, due to the proliferation of spindle cells with pleomorphism and frequent mitotic figures infiltrating the reticular dermis and subcutaneous cellular tissue, giving rise to variable size tumors with high local recurrence rates. The first-line treatment is surgical excision of the tumor with Mohs micrographic surgery among other surgical techniques for complete circumferential peripheral and deep margin assessment. However, identification of carcinogenesis mechanisms where the chromosomal translocation t (17; 22) (q22; q13) is recognized, forming the COL1A1-PDGFB fusion gene, which participates in stimulating tumor cell proliferation, allowing treatment with tyrosine kinase inhibitors such as imatinib for neoadjuvant therapy of surgically unresectable tumors and local recurrences.